Doctors ‘Right’ Baby’s Underdeveloped Heart

Until a month back, Borivli residents Kajal and Sushovan Bose had never heard about hypoplastic left heart syndrome. They found out about it only after the couple’s barely 24–hour–old son started gasping for breath at the maternity home and they had to rush to three other hospitals in a span of 12 hours in search of an answer.

By the time the young Bose went under the scalpel four days after his birth, his father had surfed the internet so thoroughly that he knew it all. "The left side of my son’s heart failed to develop during pregnancy. Some of the valves, arteries and heart chambers located on the left are too tiny in comparison to a normal heart," said 30–yearold Sushovan.

The boy became the first in western India to undergo the Norwood Procedure–known in medical circles as the final frontier in paediatric cardiac surgery. "It is one of the most complex heart surgeries. In fact, even after the procedure was developed 20 years ago, there were several ethical debates on whether such a severe procedure should be allowed at all," said Dr Suresh Rao and Dr Smrutiranjan Mohanty, the paediatric heart surgeons who operated on the child.

The operation was performed on October 13 at Kokilaben Ambani Hospital in Andheri (West), with the doctors literally reworking the heart in such a manner that blood flows despite the malformed left portion (see box).

Dr Snehal Kulkarni, the paediatric cardiologist who took care of the boy when he was first brought in, said, "Four years ago, Mumbai didn’t have a single paediatric heart centre. Children had to be taken down south for an operation. But in such a short time, Mumbai has got skills that can perform the most complex surgery."

A couple of cases of Norwood were performed in India previously, but not too successfully. However, a few months ago, Dr Colin John from Narayan Hrudlaya in Bengaluru performed the first successful Norwood operation of the country. "A Norwood operation entails not just surgery, but it needs detection of the problem in foetal stage, superlative ICU care and, not the least, financial problems," John said.

Finance is indeed a big concern. "Each operation costs about Rs 2.25 lakh and there are three operations as well as medicines to consider. Most parents are young and have little money to spare. They have to depend on the extended family and charitable organisations," said Rao.

Incidentally, the Kokilaben team has performed two Norwood operations in the last one month. "Last month, I was delivering a lecture on the ethics of the Norwood operation at a paediatric conference held in our hospital when we got the first child with a hypoplastic left heart syndrome," said Mohanty. Both the Bose baby and the second child are doing well.

Matters Of The HeartThe Disease | Hyoplastic left heart syndrome (HLHS) is a congenital defect in which the heart’s left–sided structures–the valves, ventricles and the aorta–are either narrow or have no connection between each other. Until 20 years ago, children suffering from HLHS died within 48–72 hours of birth

Mr Fix It | A treatment for HLHS was pioneered by William Norwood in Boston in the ’80s. Now, a handful of paediatric cardiac centres in the US boast of 95% success rate with the Norwood Procedure. The longest survivor (who has had all three stages of the operation) is in the teens now

New Links | The Norwood Operation involves: Attaching the base of the pulmonary artery to the aorta and inserting a shunt between a branch of the aorta and a section of the pulmonary artery Children have to undergo two more operations before the age of 10 to correct blood flow

Right On | These steps create a new pathway to bypass the underdeveloped left side of the heart. Blood goes through the pulmonary artery to the aorta and the rest of the body. Blood also flows through the mechanical shunt (connection) to the pulmonary (lung) artery. In the absence of the left ventricle, the right ventricle performs the dual function of pumping blood from the heart to the lungs and taking the blood collected from the lungs and pumping it out to the body

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